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Endocrinology

Adrenal insufficiency

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Adrenal insufficiency
Diagnosis and treatment

Adrenal insufficiency (NN, insufficiency of bark of adrenal glands, hypocorticoidism) — the clinical syndrome caused by insufficient secretion of hormones of bark of an adrenal gland as a result of disturbance of functioning of one or several links gipotalamo - pituitary and adrenal system (the tab. a hypocorticoidism). Adrenal insufficiency is subdivided into primary, being result destructions of the bark of adrenal glands, and secondary (gipotalamo-pituitary), arising owing to insufficiency secretions of AKTG by an adenohypophysis. In clinical practice primary chronic adrenal insufficiency (95% of cases) most often meets (1-XHH). The disease caused by destructive process in adrenal glands for the first time was described in 1855 by the English doctor Thomas Addison (1793-1860). Primary hypocorticoidism of an autoimmune and tubercular etiology is designated as Addison's illness.

Etiology

Are basic reasons of chronic adrenal insufficiency:

  1. Autoimmune destruction of bark of adrenal glands (autoimmune adrenalit) (98% of cases). In blood of most of patients antibodies to enzyme 21 to a hydroxylase (R450s21) are found. Besides, at 60 — 70% of patients other autoimmune diseases, most often, an autoimmune thyroiditis develop. 1-XHH autoimmune genesis is a component of autoimmune polyglandular syndromes.
  2. The adrenal tuberculosis (1-2%) develops owing to hematogenous distribution of mycobacteria. Most of patients suffers from tubercular damage of lungs.
  3. Adrenoleykodistrofiya (ALD) (1-2%) — the H-linked recessive disease at which defect of the fermental systems which are carrying out an exchange of long-chain fatty acids is inherited results from them the accumulation in the TsNS white matter and bark of adrenal glands leading to dystrophic changes. Phenotypical ALD varies from severe neurodegenerative forms to states in which clinical picture chronic adrenal insufficiency dominates.
  4. The rare reasons (coagulopathies, a dustoronny hemorrhagic heart attack of adrenal glands (Waterhouse-Frideriksen's syndrome) metastasises of tumors, a bilateral adrenalectomy, the HIV-associated complex. Various tumoral and destructive processes in gipotalamo-pituitary area are basic reasons of a secondary hypocorticoidism. Acute adrenal insufficiency (addisonichesky crisis), in most cases, has the same reasons, as chronic and represents its decompensation.

Pathogeny

At primary hypocorticoidism as a result of destruction more than 90% of bark of both adrenal glands form deficit of Aldosteronum and cortisol. Deficit of Aldosteronum leads to sodium loss, a potassium delay (hyperpotassemia) and to the progressing dehydration. A consequence of water and electrolytic frustration are changes from cardiovascular and digestive systems. Deficit of cortisol leads to decrease in adaptive opportunities of an organism, decrease in processes of a gluconeogenesis and synthesis of a glycogen. In this regard chronic adrenal insufficiency demonstrates against various physiological stresses (infections, injuries, a decompensation of the accompanying pathology). Deficit of cortisol on a negative feedback mechanism leads to increase of products of AKTG and its predecessor of a proopiomelanokortin (POMK). As a result of proteolysis of the last in addition to AKTG also the melanocyte stimulating hormone (MSH) is formed. Thus, at primary hypocorticoidism parallel to surplus of AKTG surplus of MSG which causes the brightest symptom of chronic adrenal insufficiency — hyperpegmentation of skin and mucous forms.

Because products of Aldosteronum bark of adrenal glands occur practically irrespective of effects of AKTG, at a secondary hypocorticoidism owing to a lack of AKTG deficit only of one cortisol develops, and products of Aldosteronum remain. It defines the fact that the secondary hypocorticoidism has rather easier current in comparison with primary.

Epidemiology

Primary hypocorticoidism meets frequency of 40 — 60 new cases a year on 1 million adult population. Prevalence of a secondary hypocorticoidism is unknown.

Clinical manifestations

The main clinical symptoms of primary hypocorticoidism (Addison's illness) are:

  1. Hyperpegmentation of skin and mucous. Expressiveness of hyperpegmentation corresponds to weight and prescription of process. In the beginning open parts of a body, the most subject to action of the sun — a face, a neck, hands, and also places which also normal are stronger pigmented (nipples, a scrotum, external genitals) darken. Hyperpegmentation of places of friction and visible mucous membranes has great diagnostic value. Are characteristic hyperpegmentation of palmar lines which are allocated on rather light background of surrounding fabric, darkening of places of friction with clothes, a collar, a belt. The shade of skin can vary from smoky, bronze (bronzed disease), colors of suntan, dirty skin, up to the expressed diffusion hyperpegmentation. It is rather specific, but hyperpegmentation of mucous membranes of lips, gums, cheeks, a soft and hard palate is not always expressed. Lack of a melanoderma is rather serious argument against the diagnosis of chronic adrenal insufficiency, nevertheless cases of a so-called "white addisonizm" when hyperpegmentation is almost absent meet. Against hyperpegmentation pigment-free spots (vitiligo) often are found in patients. Their size fluctuates from small, scarcely noticeable, to large, with the wrong contours, brightly allocated on the general dark background. Vitiligo is characteristic only for chronic adrenal insufficiency of autoimmune genesis.
  2. Weight loss. Its expressiveness fluctuates from moderated (3 — 6 kg) to considerable (15-25 kg), especially at initially excess body weight.
  3. General weakness, adynamy, depression, decrease in a libido. The general weakness progresses from easy, before total loss of working capacity. Patients are suppressed, sluggish, are not emotional, irritable, depressive frustration are diagnosed for a half of them.
  4. Arterial hypotension can have only orthostatic character in the beginning; patients often report about unconscious states which are provoked by various stresses. Detection at the patient of arterial hypertension — a weighty argument against the diagnosis of adrenal insufficiency though if chronic adrenal insufficiency developed against the previous idiopathic hypertensia, arterial pressure can be normal.
  5. Dispepsichesky frustration. The small appetite and diffuse pains in epigastriums, alternation of ponos and locks are most often noted. At the expressed decompensation of a disease nausea, vomiting, anorexia develop.
  6. Addiction to salty food is connected with the progressing sodium loss. In some cases it reaches the salt use in pure form.
  7. The hypoglycemia in the form of typical attacks practically does not meet, but can come to light at laboratory research.

The clinical picture of a secondary hypocorticoidism is distinguished by lack of the hyperpegmentation and symptomatology connected with deficit of Aldosteronum (arterial hypotonia, addiction to salty food, dyspepsia). Into the forefront such low-specific symptoms as the general weakness and attacks of a hypoglycemia which are described how feeling sick episodes in several hours after food act.
Clinical picture of acute adrenal insufficiency
(addisonichesky crisis) it is provided by a complex of symptoms on which dominance it is possible to allocate three clinical forms:

  1. Cardiovascular form. At this option the phenomena of an acute circulatory unefficiency dominate: pallor of the person with a Crocq's disease, a cold snap of extremities, the expressed arterial hypotonia, tachycardia, threadlike pulse, an anury, a collapse.
  2. Gastrointestinal form. On symptomatology can remind food toxicoinfection or even a condition of an acute abdomen. Abdominal pains of spastic character, nausea, pernicious vomiting, a liquid chair, a meteorism prevail.
  3. Psychological form. The headache, meningeal symptoms, spasms, focal symptomatology, nonsense, block, a stupor prevail.


 
"Mucoviscidosis   Disturbance of a salt metabolism"